Angioma Serpiginosum Clinical Presentation

Updated: May 29, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Presentation

History

Angioma serpiginosum, a rare vascular nevoid disorder due to ectatic dilation of capillaries in the papillary dermis, is found almost exclusively in females. In 2005, Sandhu and Gupta [7] reported 2 rare cases—one with familial involvement and the other with an extensive distribution of lesions. Affected individuals tend to have grouped erythematous punctate lesions on the lower limbs or buttocks.

A port-wine stain may be the first evidence of this disorder, appearing during the first few months of life. Years later, it may slowly enlarge, not by a uniform edge but rather by minute satellites ranging from copper-red to vividly red. Satellites spread into circles and gradually coalesce, producing the irregular serpiginous pattern. When the lesion resolves, it does so with a very superficial scar.

Lesions can be located anywhere on the body and have been reported in all areas except the palms and mucous membranes. [7] Late-onset ones may appear in a bandlike unilateral distribution on the chest. [12] Areas of predilection are the extremities, especially the lower extremities.

Patches are progressive and asymptomatic and rarely resolve. Rarely, patches may be extensive in distribution. [7]

Numerous small, relatively well-demarcated, round-to-oval red lagoons may be visualized with dermoscopy, which can be beneficial in the diagnosis of angioma serpiginosum. [13, 14, 15] Reflectance confocal microcopy shows multiple dilated vacular spaces in the superficial dermis and a deeper vascular plexus parallel to the skin surface. [16]

Retinal involvement has been described. [17]

Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12. [18] A 4-generation family with localized subepidermal telangiectasias following Blaschko lines (angioma serpiginosum) was described, with vascular streaks present at birth and that progressed slowly thereafter. Several family members had papillomatosis of the entire esophagus. Isolated cases of Blaschko-linear angioma serpiginosum have also been described. [19]

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Physical Examination

Angioma serpiginosum usually begins before puberty, tending to affect the lower limbs and buttocks. However, onset later in life and on other sites such as breast have been described. [20] Angioma serpiginosum is composed of reddish-purple puncta that may be as large as 1 mm. See the image below.

Angioma serpiginosum. Courtesy of DermNet New Zeal Angioma serpiginosum. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/vascular/ang-serpig2.jpg).

They are usually found grouped on the lower extremities in a serpiginous pattern. Rarely, the sole may be involved. [21] Punctate erythematous maculae on the backs of the hands, arms, and shoulders may appear following a pregnancy. [22] It may be evident in a patchy and blaschkoid distribution. [23]

Angioma serpiginosum is variably compressible. The lack of inflammation, hemorrhage, or hemosiderin pigmentation is characteristic. Diascopic pressure applied to the lesion may produce only partial emptying, with some small tufts distended by purple venous blood remaining unchanged.

Dermoscopic examination shows multiple sharply demarcated red lagoons. [24, 25] These multiple small, relatively well-demarcated, round-to-oval red lagoons may be associated with comma, hairpinlike vessels and patchy pigmentation dispersed through the background. [26]

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